Hemiatrophia faciei – en coup de sabre
Medical Review (Med. pregled), 2025, 61(2), 65-69.
S. Kordeva1, V. Broshtilova2, S. Khanom1, G. Tchernev1,3
1 Onkoderma – Clinic for Dermatology, Venereology and Dermatologic Surgery – Sofia
2 Department of Dermatology and Venereology, Military Medical Academy – Sofia
3 Department of Dermatology, Venereology and Dermatologic Surgery, Medical Institute of Ministry of Interior – Sofia
Abstract. Scleroderma, whether diffuse or localized, is a rare autoimmune connective tissue disease. Linear sclerodermia type en coup de sabre is a form of localized scleroderma that manifests on the frontoparietal scalp or paramedian forehead area. Serological testing with specific antibodies is recommended, along with histopathological verification of lesional tissue. We present a 51-year-old female with a 21-year history of linear sclerodermia, type en coup de sabre, with progressive facial hemiatrophy and cicatricial alopecia areata. The initial routine immunological assessments revealed positive results for ANA; anti-dsDNA; anti-Sm; anti-RNP protein; anti-SS-A; and anti-Scl-70. Family history of autoimmune thyroid pathology was stated. The disease was managed over the years with hydroxychloroquine, intravenous immunoglobulin infusions, and consistent hyaluronic acid injections for the facial defects. Improvement was observed after the administration of D-penicillamine.
Key words: linear scleroderma, en coup de sabre, hemiatrophia faciei, thyroid pathology, alopecia areata, D-penicillamine
Address for correspondence: Prof. Georgi Tchernev, MD, e-mail: , tel.: +359 885 588 424